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Pathophysiology and Clinical Manifestations of the β-Thalassemias

The β-thalassemia syndromes reflect deficient or absent β-globin synthesis usually owing to a mutation in the β-globin locus. The relative excess of α-globin results in the formation of insoluble aggregates leading to ineffective erythropoiesis and shortened red cell survival. A relatively high capa...

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Bibliografiske detaljer
Main Authors: Nienhuis, Arthur W., Nathan, David G.
Format: Artigo
Sprog:Inglês
Udgivet: Cold Spring Harbor Laboratory Press 2012
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3543079/
https://ncbi.nlm.nih.gov/pubmed/23209183
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1101/cshperspect.a011726
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